Bottom Line:
Previous literature has demonstrated good response with imiquimod 5% cream in patients with vulval EMPD, yet its effectiveness in primary perianal disease is unknown.We describe the case of a 40-year-old woman presenting with EMPD of the perianal region, providing detailed histological and pictoral evidence of its response to topical imiquimod 5% cream over a 16-week period, which initially resulted in remission prior to metastatic lymph node recurrence.This case demonstrates the potential for topical imiquimod cream to avoid major surgery and its associated complications in patients presenting with EMPD of the perianal region.We discuss the current evidence for treating this rare condition with medical therapy, how this case adds to current literature and possible future directions.

Mentions:
On examination, an erythematous perianal lesion was present with associated superficial excoriation (Fig. 1). An initial punch biopsy was taken (Fig. 2A), which demonstrated a unilateral well-demarcated eroded lesion. Immunohistochemical staining (Fig. 2B) showed malignant cells in the epidermis, staining positively for CK7 and EMA. The tissue stained negative for CK20 and oestrogen receptors. A diagnosis of EMPD was given.Figure 1:

Bottom Line:
Previous literature has demonstrated good response with imiquimod 5% cream in patients with vulval EMPD, yet its effectiveness in primary perianal disease is unknown.We describe the case of a 40-year-old woman presenting with EMPD of the perianal region, providing detailed histological and pictoral evidence of its response to topical imiquimod 5% cream over a 16-week period, which initially resulted in remission prior to metastatic lymph node recurrence.This case demonstrates the potential for topical imiquimod cream to avoid major surgery and its associated complications in patients presenting with EMPD of the perianal region.We discuss the current evidence for treating this rare condition with medical therapy, how this case adds to current literature and possible future directions.